- Email: [email protected]
INFORMING PATIENTS ABOUT CLINICAL DISAGREEMENT
983 treatment of
these prisoners and several doctors and nurses have been arrested and tortured. Attempts have been made to establish a medical commission, operating under one of the three human rights organisations in Morocco, but it is not yet operational. As far as we know, the Ordre National des Medecins de Maroc, which has a national council acting as watchdog on the ethical conduct of doctors (membership is obligatory), has not taken any steps to investigate the above situation. ICHP has started a invite national medical counterparts.
letter-writing campaign. Individuals can organisations to write to their Moroccan
Hogendorplaan 2, 3818 JM Amersfoort, Netherlands
right from
schools and then break the monopoly of doorstep
the of milk in delivery without
choice.
Nobody needs cows. The Japanese, with their diet of fish, cereals, and fruit, have been almost free from coronary heart disease despite much cigarette smoking and a high prevalence of hypertension and stress. It is in diets such as these that future profits lie for the food industry. In the absence of subsidies that make meat and milk products artificially cheap, this is the direction that consumption will take. Let demand, from an educated public, be the decider. Cotterlings, Ditchling,
ANNE-MARIE RAAT, van
to encourage parents to get their child’s nutrition start. Clearly an important step is to stop the distribution
Board member, Johannes Wier Foundation for Health and Human Rights (Dutch affiliate of ICHP)
Sussex BN6 8TS
RICHARD W. D. TURNER
HYPERLEXIA IN PRADER-WILLI SYNDROME
INFORMING PATIENTS ABOUT CLINICAL DISAGREEMENT
SiR,—Dr O’Dowd writes amusingly (Sept 23, p 744) about the purported inability of statistics and clinicial trials "to provide clear unequivocal answers to practising clinicians", and about the supposed superiority in medicine of case experience over epidemiological knowledge. But there is no laughter, I suspect, among hundreds of thousands of diethylstilboestrol-exposed women whose mothers were given the drug in pregnancy despite evidence from controlled trials that it had no effect on improving outcome in high-risk pregnancy.l On the other hand, George Bernard Shaw must be chuckling. In his 1911 preface to The Doctor’s Dilemma he wrote: "Doctors are no more scientific than their tailors ... [a doctor] draws disastrous conclusions from his clinical experience because he has no conception of scientific method, and believes, like any rustic, that the handling of evidence and statistics needs no expertness."2 90 La Cuesta Drive, Greenbrae, California 94904, USA 1 Chalmers TC. The impact of controlled trials Med 1976; 41: 753-58.
WILLIAM A. SILVERMAN on
the
practice of medicine
Mt
Sinai J
BEWARE OF THE COW 19 editorial Agrobureaucracy vs Health points a Government urging citizens to reduce of paradox consumption of saturated fat while subsidising farmers through the Common Agricultural Policy to produce such fats. We are not looking for a total reduction of fat intake but for a qualitative change from saturated to unsaturated fats, and this means a general move to a vegetarian type of diet. Epidemic coronary heart disease is largely due to overconsumption of meat and dairy products: there are problems with the antigenic protein, the very unbalanced fatty acid composition, the paucity of polyunsaturates, and the synergism with cholesterol. How can the change be promoted? Demand can be influenced by
SiR,—Your Aug
to
the
pricing and by education from clinicians, without the food industry. We have seen this year how a beneficial change can be accelerated by manipulation of prices. The uptake of lead-free petrol was clearly helped by the decision to tax it at a lower rate. Meat is losing popularity already, because of public concern about infection, additives, animal welfare, and its contribution to heart attacks and strokes, and this could be coupled with incentives to produce healthier foods (eg, beans). Farmers would be entitled to generous grants for adaptation. Some might opt to continue beef production in "private practice", but they would charge higher prices. A change in the perception of cow’s milk as a healthy food would be hard to achieve, but no less important. At the moment the voices realistic
"teaching" by
most
often heard are those of vested interests.
Paediatricians could be highly influential. They
are already urging more breastfeeding; and this campaign should be extended to total avoidance of cow’s milk and its products. For mothers who
cannot or will not breast feed, healthier alternatives are available. Paediatricians can explain that, after weaning, nobody needs milk at all; the only merit of cow’s milk is palatability. They are well placed
SIR,-In dyslexia people of normal intelligence cannot decode words efficiently but often have satisfactory comprehension of the material they can read. Hyperlexia is the opposite: a mentally retarded person may have good decoding skills with greatly impaired comprehension. Dyslexia in some individuals has been localised to an abnormality at chromosome 15ql 1.1,2 However, this is not a consistent fmding.3 A chromosomal basis for hyperlexia has not been identified. We have seen a left-handed man with Prader-Willi syndrome who was admitted to hospital with major depression. He had been grieving his father’s death for 2 years. Without warning he had fallen down and complained of paralysis, first on one side of his body and then on the other. He rapidly became quiet and withdrawn and began pacing the floor wringing his hands. He then had delusional feelings of guilt, stating that he was responsible for his father’s death and that the father had been poisoned. He had been bom as a breech presentation after an uncomplicated pregnancy, birthweight 2355 g. Hypotonia was observed early on and he was a poor feeder with a weak suck. He walked at 16 months, said his first word at 1 year, and was toilet trained by 2z years. He has always been in special education classes. He began to gain weight at 5 years and over the next 5 years became massively obese. He was short (height 145 cm, below 3rd percentile), obese (100 kg, above 97th percentile), and had small hands (14-6 cm, below 3rd percentile), small feet (20-8 cm, below 3rd percentile), bilateral epicanthic folds, rectangular facies, upswept low hairline anteriorly, prominent ears, hypogonadotropic hypogonadism, a bifid urethral meatus, and a history of excessive eating and rage responses to limiting food intake. His electrocardiogram was abnormal with a bifascicular and rightbundle-branch block and right axis deviation. A computerised tomographic scan and thyroid tests were normal. His EEG was mildly abnormal. Serum testosterone levels were low at 24 mg/dl. A glucose-tolerance test was consistent with type II diabetes mellitus. During his first hospital admission he presented with catatonic posturing, isolation, withdrawal, anorexia, and depression. He was treated with thiothixene and then with a course of six electroconvulsant treatments (ECT). After ECT his sleep improved, his appetite returned, and he became interpersonally involved. His mother reported that he was "back to his old self". His IQ was 75, consistent with previous testing. Evaluation of reading showed that he had decoding skills more than five grades above that predicted by IQ while comprehension was commensurate with that predicted by his IQ. He met Needleman’s criteria4 for hyperlexia, namely rapidly acquired ability to decode words, sentences, and paragraphs; onset of reading before starting school and without the benefit of reading instruction; severe intellectual and language impairment, which would ordinarily preclude the acquisition of such reading skills, present when reading skills were acquired; and much delayed reading comprehension skills that would be more consistent with intellectual and language functioning than with reading recognition skills. The Prader-Willi syndrome is associated with an interstitial deletion of chromosome 15ql l-ql3 in about 60% of patients. The same abnormality may be present in many others, but at a submicroscopic level. A study of our patient did not identify the
these prisoners and several doctors and nurses have been arrested and tortured. Attempts have been made to establish a medical commission, operating under one of the three human rights organisations in Morocco, but it is not yet operational. As far as we know, the Ordre National des Medecins de Maroc, which has a national council acting as watchdog on the ethical conduct of doctors (membership is obligatory), has not taken any steps to investigate the above situation. ICHP has started a invite national medical counterparts.
letter-writing campaign. Individuals can organisations to write to their Moroccan
Hogendorplaan 2, 3818 JM Amersfoort, Netherlands
right from
schools and then break the monopoly of doorstep
the of milk in delivery without
choice.
Nobody needs cows. The Japanese, with their diet of fish, cereals, and fruit, have been almost free from coronary heart disease despite much cigarette smoking and a high prevalence of hypertension and stress. It is in diets such as these that future profits lie for the food industry. In the absence of subsidies that make meat and milk products artificially cheap, this is the direction that consumption will take. Let demand, from an educated public, be the decider. Cotterlings, Ditchling,
ANNE-MARIE RAAT, van
to encourage parents to get their child’s nutrition start. Clearly an important step is to stop the distribution
Board member, Johannes Wier Foundation for Health and Human Rights (Dutch affiliate of ICHP)
Sussex BN6 8TS
RICHARD W. D. TURNER
HYPERLEXIA IN PRADER-WILLI SYNDROME
INFORMING PATIENTS ABOUT CLINICAL DISAGREEMENT
SiR,—Dr O’Dowd writes amusingly (Sept 23, p 744) about the purported inability of statistics and clinicial trials "to provide clear unequivocal answers to practising clinicians", and about the supposed superiority in medicine of case experience over epidemiological knowledge. But there is no laughter, I suspect, among hundreds of thousands of diethylstilboestrol-exposed women whose mothers were given the drug in pregnancy despite evidence from controlled trials that it had no effect on improving outcome in high-risk pregnancy.l On the other hand, George Bernard Shaw must be chuckling. In his 1911 preface to The Doctor’s Dilemma he wrote: "Doctors are no more scientific than their tailors ... [a doctor] draws disastrous conclusions from his clinical experience because he has no conception of scientific method, and believes, like any rustic, that the handling of evidence and statistics needs no expertness."2 90 La Cuesta Drive, Greenbrae, California 94904, USA 1 Chalmers TC. The impact of controlled trials Med 1976; 41: 753-58.
WILLIAM A. SILVERMAN on
the
practice of medicine
Mt
Sinai J
BEWARE OF THE COW 19 editorial Agrobureaucracy vs Health points a Government urging citizens to reduce of paradox consumption of saturated fat while subsidising farmers through the Common Agricultural Policy to produce such fats. We are not looking for a total reduction of fat intake but for a qualitative change from saturated to unsaturated fats, and this means a general move to a vegetarian type of diet. Epidemic coronary heart disease is largely due to overconsumption of meat and dairy products: there are problems with the antigenic protein, the very unbalanced fatty acid composition, the paucity of polyunsaturates, and the synergism with cholesterol. How can the change be promoted? Demand can be influenced by
SiR,—Your Aug
to
the
pricing and by education from clinicians, without the food industry. We have seen this year how a beneficial change can be accelerated by manipulation of prices. The uptake of lead-free petrol was clearly helped by the decision to tax it at a lower rate. Meat is losing popularity already, because of public concern about infection, additives, animal welfare, and its contribution to heart attacks and strokes, and this could be coupled with incentives to produce healthier foods (eg, beans). Farmers would be entitled to generous grants for adaptation. Some might opt to continue beef production in "private practice", but they would charge higher prices. A change in the perception of cow’s milk as a healthy food would be hard to achieve, but no less important. At the moment the voices realistic
"teaching" by
most
often heard are those of vested interests.
Paediatricians could be highly influential. They
are already urging more breastfeeding; and this campaign should be extended to total avoidance of cow’s milk and its products. For mothers who
cannot or will not breast feed, healthier alternatives are available. Paediatricians can explain that, after weaning, nobody needs milk at all; the only merit of cow’s milk is palatability. They are well placed
SIR,-In dyslexia people of normal intelligence cannot decode words efficiently but often have satisfactory comprehension of the material they can read. Hyperlexia is the opposite: a mentally retarded person may have good decoding skills with greatly impaired comprehension. Dyslexia in some individuals has been localised to an abnormality at chromosome 15ql 1.1,2 However, this is not a consistent fmding.3 A chromosomal basis for hyperlexia has not been identified. We have seen a left-handed man with Prader-Willi syndrome who was admitted to hospital with major depression. He had been grieving his father’s death for 2 years. Without warning he had fallen down and complained of paralysis, first on one side of his body and then on the other. He rapidly became quiet and withdrawn and began pacing the floor wringing his hands. He then had delusional feelings of guilt, stating that he was responsible for his father’s death and that the father had been poisoned. He had been bom as a breech presentation after an uncomplicated pregnancy, birthweight 2355 g. Hypotonia was observed early on and he was a poor feeder with a weak suck. He walked at 16 months, said his first word at 1 year, and was toilet trained by 2z years. He has always been in special education classes. He began to gain weight at 5 years and over the next 5 years became massively obese. He was short (height 145 cm, below 3rd percentile), obese (100 kg, above 97th percentile), and had small hands (14-6 cm, below 3rd percentile), small feet (20-8 cm, below 3rd percentile), bilateral epicanthic folds, rectangular facies, upswept low hairline anteriorly, prominent ears, hypogonadotropic hypogonadism, a bifid urethral meatus, and a history of excessive eating and rage responses to limiting food intake. His electrocardiogram was abnormal with a bifascicular and rightbundle-branch block and right axis deviation. A computerised tomographic scan and thyroid tests were normal. His EEG was mildly abnormal. Serum testosterone levels were low at 24 mg/dl. A glucose-tolerance test was consistent with type II diabetes mellitus. During his first hospital admission he presented with catatonic posturing, isolation, withdrawal, anorexia, and depression. He was treated with thiothixene and then with a course of six electroconvulsant treatments (ECT). After ECT his sleep improved, his appetite returned, and he became interpersonally involved. His mother reported that he was "back to his old self". His IQ was 75, consistent with previous testing. Evaluation of reading showed that he had decoding skills more than five grades above that predicted by IQ while comprehension was commensurate with that predicted by his IQ. He met Needleman’s criteria4 for hyperlexia, namely rapidly acquired ability to decode words, sentences, and paragraphs; onset of reading before starting school and without the benefit of reading instruction; severe intellectual and language impairment, which would ordinarily preclude the acquisition of such reading skills, present when reading skills were acquired; and much delayed reading comprehension skills that would be more consistent with intellectual and language functioning than with reading recognition skills. The Prader-Willi syndrome is associated with an interstitial deletion of chromosome 15ql l-ql3 in about 60% of patients. The same abnormality may be present in many others, but at a submicroscopic level. A study of our patient did not identify the